Systemic scleroderma, also called systemic sclerosis (SSc), is a multi-system autoimmune disease with a wide range of symptoms including inflammatory diseases of the muscles, swelling of the fingers and hands, microvascular abnormalities, lung disease, kidney dysfunction, cardiovascular problems, gastrointestinal malfunction, and abnormalities of the immune system that affect the connective tissue.
The disease is characterized by the breakdown of the skin, subcutaneous tissue, muscle, and internal organs due to three distinct processes: abnormalities of the immune system that lead to the production of autoantibodies and auto-immunity; micro vascular skin cell and small blood vessel fibrosis; and fibroblast dysfunction that leads to excessive collagen and other abnormal components in the skin, blood vessels and internal organs.
SSc affects about 250 per every million people in the U.S. and is three to four times more common in women than in men. It may occur at any age, but in most cases the symptoms begin during midlife.
As the disease progresses to impact major organ involvement, prognosis worsens. Pulmonary fibrosis (interstitial lung disease) and pulmonary arterial hypertension (PAH) cause more than half of SSc-related deaths.
Systemic scleroderma affects only the skin in the early stages of the disease — the skin around the mouth, nose, fingers, and other bony areas begins to thicken and becomes shiny. As it progresses, the affected areas may become tight and harder to move. Other SSc symptoms include hair loss, calcium deposits under the skin, small and dilated blood vessels under the skin’s surface, joint pain, shortness of breath, dry cough, diarrhea, constipation, difficulty swallowing, acid reflux, and bloating.
People with SSc usually experience Raynaud’s phenomenon. The condition causes spasms in the blood vessels of the fingers and toes. Extremities turn white and blue when cold or when the patient is under emotional stress.
SSc can be diagnosed through symptomatic skin changes, blood tests, chest X-rays, urinalysis, a CT scan of the lungs, and skin biopsies.
Currently, there is no cure for SSc but several therapies can help reduce disease symptoms and may slow disease progression. Palliative care is usually based on the symptoms and the need to prevent complications.
There are two subtypes of SSc. Limited systemic scleroderma features limited skin involvement with few systemic problems. Diffuse systemic scleroderma patients experience widespread skin involvement accompanied by internal organ damage.
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